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Pyogenic Arthritis, Pyoderma Gangrenosum, Acne, and Hidradenitis Suppurativa (PAPASH): A New Autoinflammatory Syndrome Associated With a Novel Mutation of the PSTPIP1 Gene

Angelo V. Marzano, MD; Valentina Trevisan, MD; Marco Gattorno, MD; Isabella Ceccherini, MD; Clara De Simone, MD; Carlo Crosti, MD
JAMA Dermatol. 2013;149(6):762-764. doi:10.1001/jamadermatol.2013.2907.
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The PASH triad (pyoderma gangrenosum, acne, and hidradenitis suppurativa) has recently been described in 2 unrelated patients as a new entity within the spectrum of autoinflammatory syndromes.1,2 PASH syndrome is similar to PAPA syndrome (pyogenic arthritis, acne, and pyoderma gangrenosum) but differs insofar as it lacks the associated arthritis and has a different genetic basis.3 PAPA syndrome is caused by mutations in the proline-serine-threonine-phosphatase protein 1 (PSTPIP1) gene (OMIM: 606347) that is involved in regulating innate immune responses, whereas no mutations have yet been detected in PASH syndrome.

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Figure 1. Clinical images of the patient in the present case. A-C, Hidradenitis suppurativa involving the intermammary fold (A), left axillae (B), and buttocks and perianal region (C). D, Pyoderma gangrenosum skin ulcers with a vegetative bed on the back. E, After healing, the ulcers leave keloidal scars.

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Figure 2. Hematoxylin-eosin–stained skin biopsy specimens taken from a pyoderma gangrenosum lesion ulcer. A, Dense dermal inflammatory infiltrate extending to the subcutis (original magnification, ×100). B, The infiltrate is mainly composed of neutrophils (original magnification, ×200).




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